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AJNR Am J Neuroradiol 21:1673–1675, October 2000
Case Report
Neurosyphilis as a Cause of Facial and Vestibulocochlear
Nerve Dysfunction: MR Imaging Features
Michelle M. Smith and James C. Anderson
loss on the right. The Distortion Product Otoacoustic Emissions test, which is a test of cochlear function, revealed bilateral cochlear dysfunction. Laboratory values on a lumbar
puncture revealed 553 nucleated cells, 90% of which were
lymphocytes and few of which were red cells. Glucose was 46
(normal range, 45–75), and protein was 236 (normal range,
15–45). Cryptococcal antigen, India ink capsule stain, serum
rheumatoid, ANCA, S-PEP, thyroid-stimulating hormone, angiotensin-converting enzyme level, routine complete blood
count, and comprehensive metabolic profiles were unremarkable. The Venereal Disease Research Laboratories (VDRL)
CSF test and polymerase chain reaction for herpes, Treponema,
and Lyme disease serologies were performed. The VDRL CSF
result was 41 positive. The remaining serologies were
negative.
An MR imaging examination was preformed on a 1.5-T MR
unit. Transverse and coronal T1-weighted spin-echo images
with a thickness of 3 mm were obtained before and after the
administration of contrast medium. The T1-weighted imaging
parameters were 450/15/4 (TR/TE/excitations). Axial T2weighted turbo spin-echo images with a thickness of 5 mm
and parameters of 5000/95/1 of the whole brain were also
obtained.
Enhancement of the intracanalicular, labyrinthine, geniculate, and tympanic segments of both seventh nerves, as well
as the cisternal segment of the right seventh and eighth cranial
nerve complex, was present (Fig 1). Enhancement of the intracanalicular portions of the vestibulocochlear nerves, the cochleae, and the meninges lining the internal auditory canals
was noted bilaterally (Fig 2). No abnormal enhancement or T2
signal was noted within the brain parenchyma, and no meningeal enhancement other than that within the internal auditory
canals was present.
Penicillin G therapy, IV administered at a rate of 4 million
units every 4 hours, was instituted. Within 1 week, the patient
had improvement in hearing and facial nerve function. The
patient was discharged to home and received 14 additional
days of IV administered antibiotic therapy. Complete recovery
of facial and vestibulocochlear nerve function was noted at the
1-month follow-up examination.
Summary: The prevalence of syphilis increased for several
decades before the mid-1990s in the United States, particularly in the southern states. We report a case of neurosyphilis causing bilateral facial and vestibulocochlear nerve
dysfunction in which the diagnosis was not initially suspected based on the patient’s demographics and history.
The MR imaging features helped to make the diagnosis in
this case and to exclude other possible causes of multiple
cranial nerve dysfunction in this patient. Hearing loss associated with neurosyphilis is one of the few treatable
forms of progressive hearing loss, and it is essential that a
diagnosis of neurosyphilis be made expeditiously.
Neurosyphilis is difficult to diagnose because of
its variable time of onset and nonspecific clinical
presentation. According to the Centers for Disease
Control, in 1995, more than 68,000 cases of syphilis were detected in adults; the highest syphilis
rates were reported in eight southern states (1).
Subsequently, the prevalence of the disease declined; however, cases remain concentrated in the
southern United States (2). Neurosyphilis may present as acute meningitis with cranial nerve dysfunction. We present the clinical and MR imaging
findings in a case of neurosyphilis causing facial
and vestibulocochlear nerve dysfunction. A high
index of suspicion and knowledge of the imaging
features are needed to make a diagnosis of neurosyphilis, and hearing loss associated with neurosyphilis can be treated (3, 4).
Case Report
A 51-year-old woman residing in Tennessee developed mild
vertigo approximately 6 weeks before admission. The patient
was able to conduct normal activities until the vertigo worsened 2 days before admission. She also noted progressive bilateral hearing loss, bilateral facial weakness, and severe headache. No history of sexually transmitted disease was elicited
at the time of admission. A physical examination at the time
of admission revealed left facial nerve paralysis and right facial
weakness. Audiograms revealed severe-to-profound sensorineural hearing loss on the left and severe sensorineural hearing
Discussion
Syphilis is a sexually transmitted disease caused
by the spirochete bacterium Treponema pallidum.
The prevalence of syphilis increased in the United
States for several decades before the mid-1990s.
The increase was secondary to the rise in unprotected homosexual activity and the increase in cases of AIDS. In 1995, the Centers for Disease Control reported that 84% of the counties in the United
States with the highest syphilis rates were located
in the southeast. Tennessee, Mississippi, and Maryland reported the highest rates, ranging from 13.5
to 15.4 cases per 100,000 (1). Higher rates of both
syphilis and HIV infection are reported in African-
Received February 17, 2000; accepted after revision April 13.
From the Department of Radiology (M.M.S.), Froedtert Memorial Lutheran Hospital, Medical College of Wisconsin, Milwaukee, WI, and the Department of Radiology (J.C.A.), Vanderbilt University Medical Center, Nashville, TN.
Presented at the 38th Annual Meeting of the American Society of Neuroradiology, Atlanta, GA, 2000.
q American Society of Neuroradiology
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SMITH
FIG 1. Axial T1-weighted MR image (450/15/4), obtained after
the administration of contrast medium, shows enhancement within the cisternal segment of the vestibulocochlear nerve complex
on the right (curved open arrow), within both internal auditory
canals, within the left cochlea (curved solid arrow), and within the
tympanic portion of the right facial nerve (small arrow). The enhancement within the internal auditory canals involves both the
nerves within the CSF and the meninges lining the canal. Enhancement is also seen within the middle turn of the right
cochlea.
FIG 2. Axial T1-weighted MR image (450/15/4), obtained after
the administration of contrast medium from a position that is
slightly superior to that of Figure 1, shows enhancement of the
labyrinthine and geniculate portions of the right facial nerve (large
arrow) and enhancement of the meninges lining both internal auditory canals (small arrows). Enhancement of the intracanalicular
segments of the seventh and eighth cranial nerve complex is
appreciated bilaterally.
Americans. The increased prevalence of syphilis in
the southeastern United States is thought to reflect
the larger African-American population existing in
that region compared with the northern and western
regions of the country. The rates of primary and
secondary syphilis have declined in the United
States since 1993; however, the disease remains
geographically concentrated in the southern United
States (2).
The infectious course of syphilis has three stages. The primary stage is characterized by the presence of a chancre, a round painless sore or ulceration at the inoculation site, and regional
adenopathy. If untreated, within 2 to 4 weeks, hematologic dissemination and the secondary form of
syphilis occur. Clinically, this manifests as a rash
on the palms of the hands and soles of the feet.
AJNR: 21, October 2000
CNS involvement can occur in the secondary stage
and may produce aseptic meningitis. Progression to
tertiary syphilis occurs after the secondary symptoms disappear. Tertiary syphilis usually does not
present until 5 years or more after the primary infection. The bacterium damages organ systems, including the CNS, cardiovascular system, bones,
joints, skin, and mucous membranes.
The CNS may become involved in any stage of
the disease from weeks to years after initial infection. Neurosyphilis is seen primarily in the tertiary
and occasionally in the secondary stages of the disease. Most patients are asymptomatic (3). The major forms of clinically symptomatic infection are
acute syphilitic meningitis, meningovascular syphilis, and parenchymal neurosyphilis (4). The neurologic symptoms produced and the temporal relationship between the primary infection and the
onset of symptoms vary between the forms of the
disease, and mixed features are common.
The parenchymatous type of neurosyphilis is
well known and results in general paresis secondary to widespread parenchymal damage and tabes
dorsalis secondary to demyelination of the posterior columns, dorsal roots, and dorsal root ganglia.
A more fulminant form of parenchymal disease, referred to as necrotizing neurosyphilis, has been reported to occur in patients with HIV (5). Meningovascular neurosyphilis is less frequent and may
produce focal neurologic deficits or global CNS
dysfunction and occurs as one of several subtypes.
Meningovascular syphilis may also present as a
subacute illness or stroke syndrome because of
small vessel arteritis (4). The symptoms appear
from months to a decade after the primary infection
and may include seizures, hemiplegia, insomnia,
personality changes, and dementia. Acute syphilitic
meningitis typically presents with headache, meningeal irritation, and confusion. Cranial nerve palsies are also common, and patients become symptomatic within 2 years of primary infection. The
symptoms and clinical presentation of the acute
meningitic and meningovascular forms of neurosyphilis can be similar (6).
The symptoms and imaging findings in our patient were most consistent with those seen in acute
syphilitic meningitis involving the basilar meninges. Meningeal irritation results in cranial neuropathies because the meningeal disease extends along
the nerves at the base of the brain. Cranial nerves
seven and eight are most frequently involved in
syphilitic basilar meningitis (3). Involvement of the
optic, oculomotor, and abducens nerves has been
reported (7).
A wide range of imaging findings may be seen
in neurosyphilis. Many cases of clinical neurosyphilis have no imaging abnormalities. In a 1989 series
of HIV-positive Haitian men with neurosyphilis,
one third had normal results of their imaging studies (5). In a 1997 study of 35 patients with documented neurosyphilis, 31% had no abnormalities
detected on their CT scans and MR images (7).
AJNR: 21, October 2000
NEUROSYPHILIS AND NERVE DYSFUNCTION
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Imaging findings in the parenchymal form of neurosyphilis may include atrophy, cerebral infarction,
white matter lesions, and enhancing nodules referred to as gummas. Meningovascular syphilis
may give rise to cerebral infarction, and the diagnosis should be considered in young patients presenting with stroke who are HIV-positive. The meningovascular and acute meningitic forms of
neurosyphilis may produce a meningoneuritis,
meningolabyrinthitis, or labyrinthitis (3). Enhancement of the leptomeninges and cranial nerves may
be seen with contrast-enhanced MR imaging. Enhancement of the vestibulocochlear nerve and labyrinth has been described in patients with luetic
labyrinthitis; however, facial nerve involvement in
addition to eighth nerve involvement is rare. Enhancement of the meninges and membranous labyrinth is likely secondary to a breakdown of the
blood-brain barrier secondary to inflammation with
a concentration of contrast medium within the abnormal membranes (8). The enhancement of the cochlea in our patient, indicative of luetic labyrinthitis, may be secondary to a spread of infection via
the endolymphatic or perilymphatic fluids. Hematogenous dissemination to the cochlea and spread
of infection via the intracanalicular portion of the
eighth cranial nerve to the cochlea are also modes
of transmission to be considered.
Facial nerve dysfunction in association with vestibulocochlear dysfunction helped to localize the
pathologic process in our patient to the cerebellopontine angle and/or temporal bone. The imaging
features in our patient included enhancement of the
seventh and eighth cranial nerves and cochlea bilaterally as well as the meninges lining the internal
auditory canals. No parenchymal abnormalities
within the brain were noted. Cranial nerve enhancement may be seen on MR images in a broad range
of pathologic entities, including neoplastic, granulomatous, infectious, posttraumatic, and idiopathic
disorders. The involvement of both the cranial
nerves and the meninges, as well as the bilateral
nature of the abnormalities, made primary neoplastic
causes unlikely in our patient. Secondary neoplastic
processes, such as leptomeningeal carcinomatosis
and lymphoma, were considered; however, the patient had no history of malignancy. In addition, the
meningeal enhancement was limited to the region of
the temporal bone without other sites of leptomeningeal involvement. Head trauma, previous radiation, and chemotherapy may produce labyrinthitis;
however, this patient had no such history. Granulomatous meningitis secondary to sarcoidosis may involve the leptomeninges and produce cranial neuropathies. Leptomeningeal sarcoidosis frequently
involves the optic chiasm and infundibulum, and
there was no such involvement in our case.
Infectious causes, including viruses such as herpes simplex virus, varicella, and herpes zoster, were
considered less likely in this patient, although these
viruses may cause facial paralysis, sensorineural
hearing loss, and vertigo. No vesicles were identified
within the external auditory canal in this patient, and
otalgia was not present, making herpes zoster infection (Ramsey-Hunt syndrome) unlikely. In addition,
it would be unlikely that meningeal or internal auditory canal enhancement would be seen in association with Ramsey-Hunt syndrome. Cytomegalovirus infection or cerebritis may cause a similar
appearance in patients with AIDS; however, this patient was HIV-negative. Bilateral facial nerve paralysis has been reported in association with Lyme disease (7); however, no history of tick bite was elicited
and Lyme serology was negative in this case. Tuberculous meningitis and fungal infection may cause
labyrinthitis; however, it is usually isolated to the
middle and inner ear and internal auditory canal involvement is unusual. The positive result of the
VDRL CSF test and rapid response to IV administered penicillin G therapy confirmed the diagnosis
of neurosyphilis in this patient, despite that a history
of sexually transmitted disease could not be elicited
from the patient. Although the VDRL CSF examination has a high specificity of 94%, the reported
sensitivity of the test for diagnosing neurosyphilis is
only 27% (9).
Conclusion
The prevalence of syphilis has increased within
the past several decades in the United States, particularly in the southeast. Neurosyphilis may occur
in the secondary and tertiary forms of the infection.
The acute meningitic and meningovascular forms
of neurosyphilis may produce cranial nerve dysfunction. Involvement of the seventh and eighth
cranial nerves results in symptoms of facial paralysis, sensorineural hearing loss, and vertigo. A high
index of suspicion and knowledge of the MR imaging features is necessary to diagnose this highly
treatable cause of progressive hearing loss and facial paralysis.
References
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Control and Prevention, Atlanta, GA. Available at: http://
www.cdc.gov/nchstp/dstd/SyphilispFacts.htm. Accessed October
4, 1999
2. STD Surveillance 1998 National Profile: Syphilis. Division of
STD Prevention, Centers for Disease Control, Atlanta, GA.
Available at: http://www.cdc.gov/nchstp/dstd/SyphilispFacts.htm.
Accessed April 18, 1999
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with AIDS. Neuroimaging Clin N Am 1997;7:(2):215–221
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